HCM care is evolving thanks to better data, new treatments
Hypertrophic cardiomyopathy (HCM) has moved from a relatively niche topic in cardiology to a major focus at several cardiovascular meetings in recent years. This trend has been driven by advances in diagnosis, imaging and treatment, according to Florian Rader, MD, director of the Hypertrophic Cardiomyopathy Center at Cedars-Sinai Hospital.
Rader said the biggest change has been new drug options for the treatment of HCM. The emergence of cardiac myosin inhibitors for obstructive HCM has particularly altered the treatment landscape, while newer data are prompting clinicians to reconsider the former standards of care with surgical myectomy or catheter-based alcohol septal ablation.
Despite the advances, HCM remains substantially underdiagnosed because people were just not looking for it for many years. This was partly because there was not much clinicians could do to treat it, and it was considered a rare disease. However, as new drug treatments became available, a much larger number of patients were identified than experts expected. Rader estimates that there may be about 1 million people with HCM in the United States, but only about 20% have been identified.
Improved imaging is helping close that gap. In addition to advances in echocardiography, CT and cardiac MRI, artificial intelligence may increasingly help identify HCM from echocardiograms and even electrocardiograms. Automated and streamlined algorithms could make recognition of the disease more efficient and help clinicians identify patients who otherwise might remain undiagnosed.
Once HCM is identified, Rader said general cardiologists can begin managing patients right away. A referral to a specialized HCM center, however, is often beneficial.
Treatment for obstructive HCM has undergone an especially dramatic change in recent years. Septal myectomy and alcohol septal ablation remain important options, particularly for patients who do not respond adequately to cardiac myosin inhibitors, but Rader said the proportion of patients requiring septal reduction therapy appears to be shrinking as newer medications demonstrate their effectiveness.
The decision between medication and an invasive procedure increasingly involves shared decision-making. Some patients may prefer septal reduction therapy as a potentially definitive intervention that avoids daily medication, while others may favor taking a pill rather than undergoing an invasive procedure with its associated risks.
“The outcomes are just really tremendous,” Rader said of cardiac myosin inhibitor therapy.
But while myosin inhibitors have made a big impact on obstructive HCM, he said there are currently no meaningful drugs available to treat non-obstructive HCM. With that in mind, there are several trials currently investigating potential new drug treatments.
Overall, considering all the change and the potential for even more new treatment options, Rader believes the world has entered a new era of HCM care.